[原著]反抗運動(Movement Oppositioniste)がみられたAtaxia-Telangiectasiaの一例

概要

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Movement oppositioniste was observed in 8-year-old female case of ataxia teleangiectasia. Her initial symptom was cerebellar ataxia at age of four. The teleangiectasia of the conjunctiva was present. Increased tendency of infections was not observed. Laboratory examination revealed decreased number of the lymphocytes, negative DNCB skin test, lowered lymphoblastic transformation by PHA test. These results indicate the disturbed cellular immunity in this patient. This typical case of ataxia teleangiectasis presented very rare symptoms of movement oppositioniste which is usually found in Wilson disease. Three types of movement oppositioniste was described. 1) Movement between nose and index finger at the time of finger nose test. 2) Movement between the index fingers like a sign of signe du bretteur. 3) Movement at the time of walking such as falling behind at the end of walking. These symptoms, above mentioned, itself is very rare findings and so far as we know, there is no description of movement oppositioniste in ataxia teleangiectasia. Since the etiology of these symptoms are still not known, wethought it worth to be reported.